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Neurofibromatosis type 1 (NF1) affects cell growth in neural tissues, resulting in neurofibromas of the internal organs, peripheral nerves and/or autonomic nerves. We describe a highly unusual case of plexiform neurofibroma presenting with lacrimal gland enlargement in an 18 year old male, which led to a diagnosis of NF1.

Background: As a subtype of neurofibromatosis, the plexiform neurofibroma is a benign, autosomally inherited disorder and predisposed to tumour formation. However, life-threatening haemorrhage into facial plexiform neurofibroma is extremely rare., Case Information: In the current study, we showed a facial plexiform neurofibroma case with massive haemorrhage in the cranio-maxillofacial region. An emergent selective angiography of the external carotid artery was performed to identify the offending artery, which was then selectively occluded by the combination of detachable coils and Onyx-34. Thus, the minimally invasive drainage surgery was successfully performed to evacuate the haematoma., Conclusion: We believe the endovascular embolization achieved its purpose by providing an initial salvage strategy for stopping active haemorrhage in plexiform neurofibroma, allowing surgeons to perform open surgery with lower complications rate.

Purpose: To report a case of diffuse orbital neurofibroma which displayed an unusual computed tomography (CT) feature., Methods: Results of ocular examination, orbital CT examination and pathological findings in a patient with a space-occupying orbital lesion are presented., Results: Orbital CT evaluation of a male patient, aged one and a half years old, revealed a right orbital lesion with a strongly negative value in Hounsfield units. The preoperative diagnosis was a dermoid cyst. An anterior orbitotomy was performed, and the surgical specimen demonstrated a typical plexiform neurofibroma with routine hematoxylin and eosin staining and immunohistochemical studies., Conclusions: Plexiform orbital neurofibromas may demonstrate a negative Hounsfield value on CT examination.